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Myasthenia Gravis, Myasthenic Syndrome, and Related Disorders 

Myasthenia Gravis, Myasthenic Syndrome, and Related Disorders
Chapter:
Myasthenia Gravis, Myasthenic Syndrome, and Related Disorders
Author(s):

Jun Kimura

DOI:
10.1093/med/9780199738687.003.0026
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date: 20 September 2021

This chapter deals with clinical and electrodiagnostic aspects of myasthenia gravis, myasthenic syndrome, and related disorders such as botulism and tick paralysis. In disorders of neuromuscular transmission, morphologic abnormalities correlate with physiologic alterations in the kinetics of acetylcholine release, which in turn diminishes twitch strength. Accumulated evidence clearly implicates the postsynaptic acetycholine receptors as the site of pathology in myasthenia gravis. In contrast, presynaptic defects of ACh release characterize the Lambert-Eaton myasthenic syndrome and botulism. Although such a dichotomy helps simplify the classification of pathogenesis, the exact physiologic or morphologic basis of various myasthenic syndromes remains unknown. These additional diseases affect the complex process of chemical transmission at different steps, as exemplified by the original case of a congenital defect of acetylcholine esterase.

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